Chromophobe renal cell carcinoma with concomitant sarcomatoid transformation and osseous metaplasia: a case report
© Tanaka et al.; licensee BioMed Central Ltd. 2013
Received: 20 August 2013
Accepted: 9 December 2013
Published: 13 December 2013
Chromophobe renal cell carcinoma is the third most common form of adult renal epithelial neoplasm. A sarcomatoid component occurs in approximately 8% of all chromophobe renal cell carcinoma cases, while metaplastic bone formation is extremely rare.
An abdominal computed tomography scan revealed a hypovascular tumor with focal calcification, measuring 2.5 × 2.3 cm, in the upper pole of the right kidney. The tumor was clinically diagnosed as a right renal cell carcinoma that showed signs of calcification, and a laparoscopic right radical nephrectomy was performed. The cut surface of the tumor was beige in color and indicated that the tumor was an extensively ossified mass. Histological analysis revealed three distinct morphological components of the tumor. The chromophobe renal cell carcinoma consisted of compact epithelial cells arranged in a nested pattern, and these were mixed with extensive areas of sarcomatoid spindle cells with marked nuclear pleomorphism and brisk mitotic activity. The tumor also contained multiple foci of metaplastic ossification.
Chromophobe renal cell carcinoma with concomitant osseous metaplasia and sarcomatoid transformation is a very rare occurrence.
KeywordsChromophobe renal cell carcinoma Sarcomatoid change Osseous metaplasia
Chromophobe renal cell carcinoma (CRCC) is the third most common form of adult renal epithelial neoplasm, accounting for 5.9% of all RCC cases , and has a better prognosis than clear cell carcinoma. The 5- and 10-year cancer-specific survival rates have been reported to be 100% and 90%, respectively , although sarcomatoid differentiation of CRCC is generally associated with a worse prognosis [2, 3]. Malignant tumors with sarcomatoid change had a 35% and 27%, 5-year disease-specific and progression-free survival, respectively . The RCC exhibits various associated secondary changes that include necrosis, hemorrhage, edema, fibrosis, and calcification. A sarcomatoid component occurs in approximately 8% of all CRCC cases , while metaplastic bone formation is extremely rare . Here, we report a unique case of CRCC with concomitant sarcomatoid transformation and massive osseous metaplasia.
The patient is alive and free from disease 12 months after surgery.
To the best of our knowledge, this is the first reported case of CRCC with concomitant osseous metaplasia and sarcomatoid transformation.
A number of recent studies concerning the prognosis of patients with CRCC have reported relatively good survival rates for this disease [1, 6], and cancer-related death occurred in 22%, 16%, and 8.6% of clear, papillary, and CRCC cases, respectively . The good survival outcomes in these cases were probably because more than 70% of patients had organ-confined disease and more than 60% of tumors were low grade .
The incidence of sarcomatoid transformation is approximately 5% in RCC , increasing to 8% in the CRCC subtype [2, 9]. Heterologous sarcomatoid components, such as chondrosarcomatous, osteosarcomatous or rhabdomyosarcomatous component have also been reported . Akhtar et al. suggested that sarcomatoid development in CRCC might be associated with its peculiar genetic profile, which makes cells prone to hyperploidization . Bruneli et al. demonstrated that both the epithelial and sarcomatoid elements in a CRCC have different genetic abnormalities and that the latter show multiple gains of chromosomes 1, 2, 6, 10, and 17 . The coexistence of both CRCC and sarcomatoid carcinoma may be due to either the dedifferentiation of the more highly differentiated chromophobe cell tumor or the coincidental development of two synchronous tumors . The former model is more widely accepted as it is based on the evolution of renal carcinoma into a spindle cell population. This sarcomatoid change may be the result of extensive chromosomal rearrangement, leading to identical spindle morphology .
Sarcomatoid CRCC is a more aggressive neoplasm and has a very poor prognosis compared with classic chromophobe carcinoma . The presence of a sarcomatoid component has been reported to be associated with an increased risk of metastasis and an unfavorable prognosis [2, 9, 14]. Previously reported cases of CRCC with sarcomatoid differentiation have shown distant organ metastases to sites including the bone, liver, lung, and lymph nodes .
RCCs exhibit a number of changes such as hemorrhage, necrosis, fibrosis, and hyalinization. However, although calcification is a well-recognized feature of renal tumors, they are rarely ossified. Daniel reported that 10.3% of RCCs had calcified foci, based on a review of 2,709 renal masses at the Mayo Clinic . Moreover, reports of histopathologically confirmed osseous metaplasia or bone formation within RCC are rare . The presence of ossification in CRCC, however, was noted in four previously reported cases .
The mechanism of ossification is unclear, although it might involve a metaplastic or reparative response either in the tumor or in the surrounding tissue, the production of bone by tumor cells, or the ossification of a preexisting mucin or calcium deposit . Osseous metaplasia may occur secondary to ischemia, necrosis, or inflammation in the tumor or surrounding tissues .
The prognostic significance of ossification in CRCC remains unclear because of the limited number of cases reported to date. However, several studies demonstrated that ossification is a significant prognostic marker for patients with RCC [4, 17] and is usually representative of an early stage without invasion or metastasis . Therefore, RCC with osseous metaplasia implies a more favorable prognosis, although this is contradicted by some reports, suggesting that ossification is actually associated with high-grade tumors and a poor prognosis .
In conclusion, CRCC with concomitant osseous metaplasia and sarcomatoid transformation is a very rare occurrence. It is important to recognize that this unusual variant of renal cancer has the potential to behave aggressively and to metastasize.
Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
YT: Resident. TK: Associate professor. SH: Lecturer. YH: Associate professor. CO: Professor and Chairman. Department of Urology, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
Chromophobe renal cell carcinoma
Renal cell carcinoma.
- Amin MB, Amin MD, Tamboli P, Javidan J, Stricker H, De-Peralta Venturina M, Deshpande A, Menon M: Prognostic impact of histological subtyping of adult renal epithelial neoplasms. Am J Surg Pathol. 2002, 26: 281-291. 10.1097/00000478-200203000-00001.View ArticlePubMedGoogle Scholar
- Amin MB, Paner GP, Alvarado-Cabrero I, Young AN, Stricker HJ, Lyles RH, Moch H: Chromophobe renal cell carcinoma: histomorphologic characteristics and evaluation of conventional pathologic prognostic parameters in 145 cases. Am J Surg Pathol. 2008, 32: 1822-1834. 10.1097/PAS.0b013e3181831e68.View ArticlePubMedGoogle Scholar
- Klatte T, Han KR, Said JW, Böhm M, Allhoff EP, Kabbinavar FF, Belldegrun AS, Pantuck AJ: Patholobiology and prognosis of chromophobe renal cell carcinoma. Urol Oncol. 2008, 26: 604-609. 10.1016/j.urolonc.2007.07.015.View ArticlePubMedGoogle Scholar
- Kefeli M, Yildiz L, Aydin O, Kandemir B, Yilmaz AF: Chromophobe renal cell carcinoma with osseous metaplasia containing fatty bone marrow element: a case report. Pathol Res Practice. 2007, 203: 749-752. 10.1016/j.prp.2007.06.003.View ArticleGoogle Scholar
- Sobin LH, Gospodarowicz MK, Wittekind C: TNM classification of malignant tumors. 2009, New York: Wiley-Liss, 7Google Scholar
- Beck SD, Patel MI, Snyder ME, Kattan MW, Motzer RJ, Reuter VE, Russo P: Effect of papillary and chromophobe cell type on disease-free survival after nephrectomy for renal cell carcinoma. Ann Surg Oncol. 2004, 11: 71-77. 10.1007/BF02524349.View ArticlePubMedGoogle Scholar
- Cindolo L, de la Taille A, Schips L, Zigeuner RE, Ficarra V, Artibani W, Gallo A, Salzano L, Patard JJ: Chromophobe renal cell carcinoma: comprehensive analysis of 104 cases from multicenter European database. Urology. 2005, 65: 681-686. 10.1016/j.urology.2004.11.005.View ArticlePubMedGoogle Scholar
- Cheville JC, Lohse CM, Zincke H, Weaver AL, Leibovich BC, Frank I, Blute ML: Sarcomatoid renal cell carcinoma: an examination of underlying histologic subtype and an analysis of associations with patient outcome. Am J Surg Oncol. 2004, 28: 435-441.Google Scholar
- Gira FA, Barbieri A, Fellegara G, Zompatori M, Corradi D: Dedifferentiated chromophobe renal cell carcinoma with massive osteosarcoma-like divergent differentiation: a singular entity in the spectrum of retroperitoneal calcifying tumors. Int J Surg Pathol. 2010, 18: 419-423.PubMedGoogle Scholar
- Quiroga-Garza G, Khurana H, Shen S, Ayala AG, Ro JY: Sarcomatoid chromophobe renal cell carcinoma with heterologous sarcomatoid elements: a case report and review of the literature. Arch Pathol Lab Med. 2009, 133: 1857-1860.PubMedGoogle Scholar
- Akhtar M, Tulbah A, Kardar AH, Ali MA: Sarcomatoid renal cell carcinoma: the chromophobe connection. Am J Surg Pathol. 1997, 21: 1188-1195. 10.1097/00000478-199710000-00009.View ArticlePubMedGoogle Scholar
- Brunelli M, Gobbo S, Cossu-Rocca P, Cheng L, Hes O, Delahunt B, Pea M, Bonetti F, Mina MM, Ficarra V, Chilosi M, Eble JN, Menestrina F, Martignoni G: Chromosomal gains in the sarcomatoid transformation of chromophobe renal cell carcinoma. Mod Pathol. 2007, 20: 303-309. 10.1038/modpathol.3800739.View ArticlePubMedGoogle Scholar
- Parada D, Peña K, Moreira O: Sarcomatoid chromophobe renal cell carcinoma: a case report and review of the literature. Arch Esp Urol. 2006, 2: 209-214.Google Scholar
- Cserni G, Kovács BR, Tarján M, Sápi Z, Domján Z, Szabó Z: Sarcomatoid renal cell carcinoma with foci of chromophobe carcinoma. Pathol Oncol Res. 2002, 8: 142-144. 10.1007/BF03033725.View ArticlePubMedGoogle Scholar
- Daniel WW, Hartman GW, Witten DM, Farrow GM, Kelalis PP: Calcified renal masses: a review of ten years experience at the Mayo clinic. Radiology. 1972, 103: 503-508.View ArticlePubMedGoogle Scholar
- Haddad FS, Shah IA, Manné RK, Costantino JM, Somsin AA: Renal cell carcinoma insulated in the renal capsule with calcification and ossification. Urol Int. 1993, 51: 97-101. 10.1159/000282522.View ArticlePubMedGoogle Scholar
- Sostre G, Johnson JF, Cho M: Ossifying renal cell carcinoma. Pediatr Radiol. 1998, 28: 458-460. 10.1007/s002470050382.View ArticlePubMedGoogle Scholar
- Bielsa O, Lloreta J, Arango O, Serrano S, Gelabert-Mas A: Bone metaplasia in a case of bilateral renal cell carcinoma. Urol Int. 2001, 66: 55-56. 10.1159/000056568.View ArticlePubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2490/13/72/prepub
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